Spontaneous isolated superior mesenteric artery dissection is a very rare vascular disease that involves the superior mesenteric artery or its branches, with an incidence as low as 0.08%. The majority of cases occur in patients of Asian descent. Due to advances in imaging modalities, particularly abdominal computed tomography angiography, the diagnosis of this disease has been increasing. Herein, we present a rare case of spontaneous isolated superior mesenteric artery dissection with thrombosis in a young male patient with no past medical history. The importance of this disease as a differential diagnosis for acute abdominal pain is emphasized.